Sleep-related breathing disorders in facioscapulohumeral dystrophy.
Fascioscapulohumeral dystrophy
Nocturnal hypoventilation
Non-invasive ventilation
Sleep-disordered breathing
Transcutaneous capnometry
Journal
Sleep & breathing = Schlaf & Atmung
ISSN: 1522-1709
Titre abrégé: Sleep Breath
Pays: Germany
ID NLM: 9804161
Informations de publication
Date de publication:
Sep 2019
Sep 2019
Historique:
received:
12
01
2019
accepted:
08
04
2019
revised:
04
04
2019
pubmed:
27
4
2019
medline:
3
11
2020
entrez:
27
4
2019
Statut:
ppublish
Résumé
Severe manifestations of facioscapulohumeral dystrophy (FSHD) may be associated with sleep-disordered breathing (SDB), including obstructive sleep apnea (OSA) and nocturnal hypoventilation (NH), but prevalence data are scarce. In patients with respiratory muscle weakness, detection of NH can be facilitated by transcutaneous capnometry, but respective data derived from FSHD patients have not yet been published. We collected sleep studies and capnometry recordings from 31 adult patients with genetically confirmed FSHD who were admitted to our sleep laboratory for first-ever evaluation of sleep-related breathing. Indications for admission included non-restorative sleep, morning headache, or excessive daytime sleepiness. In addition, sleep studies were initiated if symptoms or signs of respiratory muscle weakness were present. Thirty-one subjects with insomnia served as controls for comparison of respiratory measures during sleep. In the FSHD group, 17/31 (55%) patients showed OSA and 8 (26%) had NH. NH would have been missed in 7/8 patients if only oximetry criteria of hypoventilation had been applied. Capnography results were correlated with disease severity as reflected by the Clinical Severity Score (CSS). Non-invasive ventilation (NIV) was started in 6 patients with NH and 3 individuals with OSA. Nocturnal continuous positive airway pressure was administered to 2 patients, and positional therapy was sufficient in 4 individuals. In patients initiated on NIV, nocturnal gas exchange already improved in the first night of treatment. SDB is common in adult patients with FSHD complaining of sleep-related symptoms. It may comprise OSA, NH, and most often, the combination of both. Sleep-related hypercapnia is associated with disease severity. Transcutaneous capnometry is superior to pulse oximetry for detection of NH.
Identifiants
pubmed: 31025273
doi: 10.1007/s11325-019-01843-1
pii: 10.1007/s11325-019-01843-1
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
899-906Commentaires et corrections
Type : CommentIn
Type : CommentIn
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